ABCD1 蛋白, Human (Cell-Free, His)

ABCD1, Human (His) is one of the four ATP-binding cassette (ABC) proteins in subfamily D. Dysfunction of ABCD1 causes neurodegenerative disorder X-linked adrenoleukodystrophy (X-ALD).

ALDP 包含六个假定的跨膜结构域 (TMD) 和一个核苷酸结合结构域 (NBD)。ALDP 在游离多核糖体上合成，翻译后转运至过氧化物酶体，并插入膜中。在此过程中，ALDP 与 Pex19p 相互作用，Pex19p 是一种用于 PMP 细胞内运输的伴侣蛋白，该复合物靶向过氧化物酶体膜上的 Pex3p，然后 ALDP 插入膜中。整合到膜中后，ALDP被认为主要形成同二聚体，并部分与其他过氧化物酶体ABC蛋白形成异二聚体，例如70-kDa过氧化物酶体膜蛋白(PMP70/ABCD3)和肾上腺脑白质营养不良相关蛋白(ALDRP/ABCD2)。ABCD1 功能障碍会导致神经退行性疾病 X 连锁肾上腺脑白质营养不良 (X-ALD)，其特征是过氧化物酶体 β-氧化受损导致 VLCFA 异常积累^[1][2]。

Human

E. coli Cell-free

C-10*His

P33897 (M1-T745)

215  [NCBI]

MPVLSRPRPWRGNTLKRTAVLLALAAYGAHKVYPLVRQCLAPARGLQAPAGEPTQEASGVAAAKAGMNRVFLQRLLWLLRLLFPRVLCRETGLLALHSAALVSRTFLSVYVARLDGRLARCIVRKDPRAFGWQLLQWLLIALPATFVNSAIRYLEGQLALSFRSRLVAHAYRLYFSQQTYYRVSNMDGRLRNPDQSLTEDVVAFAASVAHLYSNLTKPLLDVAVTSYTLLRAARSRGAGTAWPSAIAGLVVFLTANVLRAFSPKFGELVAEEARRKGELRYMHSRVVANSEEIAFYGGHEVELALLQRSYQDLASQINLILLERLWYVMLEQFLMKYVWSASGLLMVAVPIITATGYSESDAEAVKKAALEKKEEELVSERTEAFTIARNLLTAAADAIERIMSSYKEVTELAGYTARVHEMFQVFEDVQRCHFKRPRELEDAQAGSGTIGRSGVRVEGPLKIRGQVVDVEQGIICENIPIVTPSGEVVVASLNIRVEEGMHLLITGPNGCGKSSLFRILGGLWPTYGGVLYKPPPQRMFYIPQRPYMSVGSLRDQVIYPDSVEDMQRKGYSEQDLEAILDVVHLHHILQREGGWEAMCDWKDVLSGGEKQRIGMARMFYHRPKYALLDECTSAVSIDVEGKIFQAAKDAGIALLSITHRPSLWKYHTHLLQFDGEGGWKFEKLDSAARLSLTEEKQRLEQQLAGIPKMQRRLQELCQILGEAVAPAHVPAPSPQGPGGLQGAST

Approximately 84.9 kDa

Greater than 90% as determined by reducing SDS-PAGE.

<1 EU/μg, determined by LAL method.

• [1]. Takahashi N, et, al. Adrenoleukodystrophy: subcellular localization and degradation of adrenoleukodystrophy protein (ALDP/ABCD1) with naturally occurring missense mutations. J Neurochem. 2007 Jun;101(6):1632-43.  [Content Brief]

  [2]. Kawaguchi K, et, al. Acyl-CoA thioesterase activity of peroxisomal ABC protein ABCD1 is required for the transport of very long-chain acyl-CoA into peroxisomes. Sci Rep. 2021 Jan 26;11(1):2192.  [Content Brief]